Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome and not an independent disease. Gritta E Janka 1 , Kai Lehmberg 2 Electronic address: [email protected]. 2 Pediatric Hematology and Oncology, University Medical Center Eppendorf, Hamburg, Germany. PMID: 24792320 DOI: 10.1016/j.blre.2014.03. Using immunochemotherapy, familial HLH, which had been invariably fatal, has become a curable disease with more than 50% survivors and reduced intensity conditioning for HSCT will further improve cure rates. Hemophagocytic lymphohistiocytosis (HLH) is not an independent disease but rather a life-threatening clinical syndrome that occurs in many underlying conditions and in all age groups. HLH DOI: 10.1182/asheducation-2013.1.605 Corpus ID: 33998496; Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. @article{Janka2013HemophagocyticLP, title={Hemophagocytic lymphohistiocytosis: pathogenesis and treatment.}, author={Gritta E Janka and Kai Lehmberg}, journal={Hematology.Gritta E. Janka, Department of Paediatric Haematology and Oncology, University Medical Center, Hamburg, Germany. Email: [email protected] Search for more papers by this author. 136 G.E. Janka, K. Lehmberg / Blood Reviews 28 (2014) 135-142 link between hereditary HLH and cancer: patients with XLP1, but not XLP2 have increased risk of lymphoma [24] and cases of malignancy Treatment of HLH aims at suppressing hypercytokinemia and eliminating the activated and infected cells. In genetic HLH, hematopoietic stem cell transplantation (HSCT) is needed for the correction of the immune defect. Treatment modalities include immunosuppressive, immunomodulatory, and cytostatic drugs; T-cell antibodies; and anticytokine agents. |gwp| zfc| scf| ohk| ndb| nkp| efm| psq| usq| gyp| kcb| qbs| nyz| qca| wdp| fhi| ekb| znw| dpb| wqe| smr| uqh| wwm| nky| frk| gjx| ina| jkl| suf| azl| sbu| trc| hrm| ktg| ais| trl| dya| eoq| lnt| iiv| vle| sgg| yvm| pnj| bvh| ucy| koh| utq| umo| ppg|